‘’The completed investigation did not support the patient's having had
extended travel to European countries, including the United Kingdom, or travel
to Saudi Arabia. The specific overseas country where this patient’s infection
occurred is less clear largely because the investigation did not definitely link
him to a country where other known vCJD cases likely had been infected.’’

CDC and the Texas Department of State Health Services (DSHS) have completed
the investigation of the recently reported fourth vCJD case in the United
States. It confirmed that the case was in a US citizen born outside the Americas
and indicated that the patient's exposure to the BSE/vCJD agent most likely
occurred before he moved to the United States; the patient had resided in
Kuwait, Russia and Lebanon. The completed investigation did not support the
patient's having had extended travel to European countries, including the United
Kingdom, or travel to Saudi Arabia. The specific overseas country where this
patient’s infection occurred is less clear largely because the investigation did
not definitely link him to a country where other known vCJD cases likely had
been infected.

Blood meal used to make cattle feed was recalled because it was cross-
contaminated with prohibited bovine meat and bone meal that had been
manufactured on common equipment and labeling did not bear cautionary BSE
statement.

*** why do we not want to do TSE transmission studies on chimpanzees $

5. A positive result from a chimpanzee challenged severly would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were
exposed to the infectious agents only by their nonforced consumption of known
infectious tissues. The asymptomatic incubation period in the one monkey exposed
to the virus of kuru was 36 months; that in the two monkeys exposed to the virus
of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the
two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively.
Careful physical examination of the buccal cavities of all of the monkeys failed
to reveal signs or oral lesions. One additional monkey similarly exposed to kuru
has remained asymptomatic during the 39 months that it has been under
observation.

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie
by natural feeding to squirrel monkeys that we have reported provides further
grounds for concern that scrapie-infected meat may occasionally give rise in
humans to Creutzfeldt-Jakob disease.

Recently the question has again been brought up as to whether scrapie is
transmissible to man. This has followed reports that the disease has been
transmitted to primates. One particularly lurid speculation (Gajdusek 1977)
conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and
transmissible encephalopathy of mink are varieties of a single "virus". The U.S.
Department of Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed for human
or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is
emphasised by the finding that some strains of scrapie produce lesions identical
to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety of laboratory
personnel requires prompt attention. Second, action such as the "scorched meat"
policy of USDA makes the solution of the acrapie problem urgent if the sheep
industry is not to suffer grievously.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes
of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey
(Macaca fascicularis) with an incubation period of more than 5 yr from the time
of intracerebral inoculation of scrapie-infected mouse brain. The animal
developed a chronic central nervous system degeneration, with ataxia, tremor and
myoclonus with associated severe scrapie-like pathology of intensive astroglial
hypertrophy and proliferation, neuronal vacuolation and status spongiosus of
grey matter. The strain of scrapie virus used was the eighth passage in Swiss
mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral
passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton,
Berkshire).

IT is of my opinion, that the OIE and the USDA et al, are the soul reason,
and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion
diseases, including typical and atypical BSE, typical and atypical Scrapie, and
all strains of CWD, and human TSE there from, spreading around the globe.

I have lost all confidence of this organization as a regulatory authority
on animal disease, and consider it nothing more than a National Trading
Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i
said before, OIE should hang up there jock strap now, since it appears they will
buckle every time a country makes some political hay about trade protocol,
commodities and futures. IF they are not going to be science based, they should
do everyone a favor and dissolve there organization.

JUST because of low documented human body count with nvCJD and the long
incubation periods, the lack of sound science being replaced by political and
corporate science in relations with the fact that science has now linked some
sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of
CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call
for this organization to be dissolved. ...

REPORT OF THE COMMITTEE 344 Ewes were experimentally inoculated with brain
homogenate obtained from a U.S. sheep with clinical Nor98-like scrapie.

Recipient ewes are bred annually to examine the placenta for evidence of a
transmissible agent. Placentas shed 2009-2013 were negative.

*** In 2013, one recipient ewe developed an unrelated disease. At
postmortem examination, abundant accumulation of PrPSc was observed only in the
cerebellum of this ewe with much less accumulation in the hindbrain obex. This
confirms that initial inoculation of these ewes has been successful. Monitoring
continues in the remaining ewes of this study.

USA, NORTH AMERICA, MBM (or any potential TSE prion disease) EXPORTS TO THE
WORLD (?) [protected by the BSE MRR policy] $$$

IN my opinion, from the following risk factors i will post below, and the
fact that the OIE and the USDA systematically did away with the BSE GBR system
for the BSE MRR system, for the legal trading all strains of TSE globally, and
the ramifications there from (BSE MRR), MY confidence level of any TSE
regulatory risk assessment is 0...that is ZERO CONFIDENCE LEVEL IN ANY REGARDS
TO THE TSE PRION DISEASES AKA MAD COW DISEASE. The BSE MRR regulations were set
up to fail, and make legal the trading of all strains of TSE prion disease
globally. the consumers were hung out to dry around the globe, and the
ramifications there from will be long and costly thanks to the OIE and the USDA
et al. ...TSS

Scientific Report of the European Food Safety Authority on the Assessment
of the Geographical BSE-Risk (GBR) of United States of America (USA)

Question N° EFSA-Q-2003-083

Adopted July 2004

Summary of scientific report The European Food Safety Authority and its
Scientific Expert Working Group on the Assessment of the Geographical Bovine
Spongiform Encephalopathy (BSE) Risk (GBR) were asked by the European Commission
(EC) to provide an up-to-date scientific report on the GBR in the United States
of America, i.e. the likelihood of the presence of one or more cattle being
infected with BSE, pre-clinically as well as clinically, in USA. This scientific
report addresses the GBR of USA as assessed in 2004 based on data covering the
period 1980-2003. The BSE agent was probably imported into USA and could have
reached domestic cattle in the middle of the eighties. These cattle imported in
the mid eighties could have been rendered in the late eighties and therefore led
to an internal challenge in the early nineties. It is possible that imported
meat and bone meal (MBM) into the USA reached domestic cattle and leads to an
internal challenge in the early nineties. A processing risk developed in the
late 80s/early 90s when cattle imports from BSE risk countries were slaughtered
or died and were processed (partly) into feed, together with some imports of
MBM. This risk continued to exist, and grew significantly in the mid 90’s when
domestic cattle, infected by imported MBM, reached processing. Given the low
stability of the system, the risk increased over the years with continued
imports of cattle and MBM from BSE risk countries. EFSA concludes that the
current GBR level of USA is III, i.e. it is likely but not confirmed that
domestic cattle are (clinically or pre-clinically) infected with the BSE-agent.
As long as there are no significant changes in rendering or feeding, the
stability remains extremely/very unstable. Thus, the probability of cattle to be
(pre-clinically or clinically) infected with the BSE-agent persistently
increases.

Annex to the EFSA Scientific Report (2004) 3, 1-17 on the Assessment of the
Geographical BSE Risk of USA - 1 - European Food Safety Authority Scientific
Expert Working Group on GBR Working Group Report on the Assessment of the
Geographical BSE-Risk (GBR) of UNITED STATES OF AMERICA 2004

Annex to the EFSA Scientific Report (2004) 3, 1-17 on the Assessment of the
Geographical BSE Risk of USA - 7 - 2.3

Overall assessment of the external challenge

The level of the external challenge that has to be met by the BSE/cattle
system is estimated according to the guidance given by the SSC in its final
opinion on the GBR of July 2000 (as updated in January 2002). Live cattle
imports: In total the country imported 2038 (other sources) or 1128 (CD) live
cattle from BSE risk countries other than Canada, of which 327 (other sources)
or 323 (CD) came from the UK. From Canada the imports were >500,000 animals
per year. The numbers shown in table 1 are the raw import figures and are not
reflecting the adjusted imports for the assessment of the external challenge.
Broken down to 5 year periods the resulting external challenge is as given in
table 3. This assessment takes into account the different aspects discussed
above that allow to assume that certain imported cattle did not enter the
domestic BSE-cattle system, i.e. were not rendered into feed. In the case of the
USA, all the animals for which tracing information showed that they were not
rendered were excluded from the external challenge.

MBM imports:

In total the country imported 689 tons MBM (CD) or 2,230 tons MBM (other
sources) from BSE risk countries other than Canada, of which 5 tons (CD) or 101
tons (other sources) were exported from the UK (UK export data). From Canada,
the imports were about 30 000 tons per year. The numbers shown in table 2 are
the raw import figures and are not reflecting the adjusted imports for the
assessment of the external challenge. Broken down to 5 year periods the
resulting external challenge is as given in table 3. This assessment takes into
account the different aspects discussed above that allow to assume that certain
imported MBM did not enter the domestic BSE/cattle system or did not represent
an external challenge for other reasons. As it was illegal to export mammalian
MBM from UK since 27/03/1996, exports indicated after that date should only have
included non-mammalian MBM. In the case of the USA imported MBM from UK in 1989
and between 1997 and 1999 was not taken into account.

Feeding Use of MBM in cattle feed

• Until 1997 ruminant MBM (RMBM) could legally be included in cattle feed
and was indeed commonly fed to cattle of different age and type. Prior to the
feed ban the US authorities estimated that 10% of all MBM would deliberately
have been fed to cattle. Feed bans

• A ban to feed (several types of) MMBM to ruminants was put in place in
August 1997. Derogation from the ban was granted for pure porcine and equine
protein (MBM) coming from designated (single species) rendering plants. This
MMBM might still be fed to cattle. Therefore this feed ban is a ruminant to
ruminant ban.

• It is planned to prohibit the use of all mammalian and poultry protein in
ruminant feed and prohibiting materials from non-ambulatory disabled cattle and
dead stock from use in all animal feed.

Conclusion on the ability to avoid recycling

• Before 1997, US system would not have been able to avoid recycling of the
BSEagent to any measurable extent. If the BSE-agent was introduced into the feed
chain, it could have reached cattle.

• After the introduction of the 1997 ban in August 1997, the ability to
avoid recycling of BSE-infectivity was somewhat improved. However, the rendering
of ruminant material (including SRM and fallen stock) is inadequate (non
pressurized), and cross-contamination potentials of cattle feed with other feeds
remain.

• Therefore, the system is still unable to avoid recycling of
BSE-infectivity if already present in the system or incoming.

Feeding

Until August 1997, RMBM was legally fed to cattle. Feeding was therefore
"not OK". In August 1997 an RMBM-ban was introduced but feeding of non-ruminant
MBM to cattle remained legal as well as feeding of RMBM to non-ruminant animals
(farm animals and pets). An RMBM ban is difficult to maintain, as only labels
can distinguish the various MMBMs. This makes control of the feed ban very
difficult because analytical differentiation between ruminant and non-ruminant
MBM is difficult if not impossible.

Due to the highly specialised production system in the USA, various
mammalian MBM streams can be separated. Such a feed ban would therefore be
assessed as "reasonably OK", for all regions where this highly specialised
system exists. However, several areas in the USA do have mixed farming and mixed
feed mills, and in such regions an RMBM ban would not suffice. Additionally,
official controls for cattle feeds to control for compliance with the ban
started in 2002. Thus, for the whole country, the assessment of the feeding
after 1997 remains "not OK", but improving.

Rendering

The rendering industry is operating with processes that are not known to
reduce

infectivity. It is therefore concluded that rendering was and is "not
OK".

SRM-removal

SRM were and are still rendered for feed, as are (parts of) the fallen
stock. SRMremoval

is therefore regarded as "not OK".

BSE-surveillance

Before 1989, the ability of the system to identify (and eliminate)
BSE-cases was

limited. Since 1990 this ability is improved, thanks to a specific
(passive) BSE

surveillance. The initiated introduction of active surveillance in risk
populations

should improve the system significantly.

On the basis of the available information, it has to be concluded that the
country's

BSE/cattle system was extremely unstable until today, i.e., it would have
recycled and

amplified BSE-infectivity very fast, should it have entered the system. The
stability of

the BSE/cattle system in the USA overtime is as given in table 4.

The present assessment modifies the stability assessment of the previous
GBR report

in 2000 mainly due to a different perception of the impact of BSE
surveillance on

stability and of the efficiency of the RMBM feed ban.

Interaction of stability and external challenge in the USA

Period Stability External Challenge Internal challenge

1980 to

1985

1986 to

1990

Moderate Possibly present

1991 to 1995

Very high

1996 to

2000

2001 to

2003

Extremely unstable Extremely high Likely to be present and growing

5. CONCLUSION ON THE GEOGRAPHICAL BSE-RISK

5.1 The current GBR as function of the past stability and challenge

• The current geographical BSE risk (GBR) level is III, i.e. it is likely
but not

confirmed that domestic cattle are (clinically or pre-clinically) infected
with the

BSE-agent.

Note1: It is also worth noting that the current GBR conclusions are not
dependent on

the large exchange of imports between USA and Canada. External challenge
due to

exports to the USA from European countries varied from moderate to high.
These

challenges indicate that it was likely that BSE infectivity was introduced
into the

EFSA publishes Geographical BSE-Risk (GBR) assessments for Australia,
Canada, Mexico, Norway, South Africa, Sweden and the United States of
America

Communiqué de presse 20 août 2004

The European Food Safety Authority (EFSA) has issued today seven up-to-date
scientific reports on the Geographical Bovine Spongiform Encephalopathy (BSE)
Risk (GBR) assessments for Australia, Canada, Mexico, Norway, South Africa
Sweden and the United States of America. While Australia’s GBR level I (i.e.
presence of BSE in domestic cattle is highly unlikely) is maintained, that of
Norway has been raised to level II (presence of BSE unlikely but not excluded),
Sweden remains at GBR level II and those of Canada and the United States have
been raised to level III (presence of BSE likely but not confirmed, or confirmed
at a lower level) following a new assessment taking into account the most recent
evidence. EFSA’s Scientific Expert Working Group on geographic BSE risk
assessment also evaluated the status of Mexico and South Africa which were
classified as level III.

In March, two trading partners agreed to resume imports of U.S. beef for
the first time since December 2003. Ecuador and Sri Lanka were among a handful
of nations that had never reopened after the first U.S. case of BSE, but both
are now accepting U.S. beef.

Ecuador offers strong potential for U.S. beef offal sales

Exports to Ecuador face very few restrictions, with muscle cuts and offal
items from cattle of all ages now eligible.

“That’s a huge bonus in a market where we expect to export a significant
volume of offal products,” says Cheyenne Dixon, U.S. Meat Export Federation
(USMEF) technical services manager.

The only significant constraint involves beef derived from cattle imported
from Canada, in which case the animal must be in the U.S. for 60 days prior to
slaughter.

“We hope this restriction is temporary, and it is a point on which the U.S.
and Ecuadorian governments continue to negotiate,” Dixon explains. “But products
derived from all domestic cattle are eligible, as well as beef from cattle
imported from Mexico.”

In addition to providing high-quality beef cuts for Ecuador’s hotel and
restaurant sectors, USMEF expects U.S. exporters to find success with variety
meat items such as beef tripe, livers and hearts. Two introductory seminars for
Ecuadorian importers are planned later this month – one in the capital city of
Quito and one in Ecuador’s largest city, Guayaquil.

According to the Global Trade Atlas, Ecuador imported 1,006 metric tons
(mt) of beef last year at a value of $4.5 million – down about 10% from 2012.
Chile was Ecuador’s leading beef supplier, followed by Uruguay.

Sri Lanka's tourism push offers potential for U.S. beef products

Located off the southeastern coast of India, Sri Lanka was plagued by civil
war and ongoing political conflict until 2009. The country was also devastated
by a deadly tsunami in 2004. But recently, Sri Lankans have enjoyed a much more
peaceful existence, which has led to economic growth and a revitalized tourism
sector.

“Sri Lanka has ambitious plans to make it onto the ‘A list’ of Asian
tourist destinations, in line with Bali (Indonesia) and several locations in
Thailand,” says Joel Haggard, USMEF senior vice president for the Asia
Pacific.

Haggard also notes that the capital city of Colombo, which has a
greater-metropolitan population of approximately 1.5 million people, has several
modern supermarket chains that currently rely mostly on domestic products.

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“Sri Lanka’s retail sector is expanding rapidly and there are certainly
growth opportunities there for U.S. beef,” Haggard says.

Recently a group of veterinary officials from Sri Lanka’s Central
Department of Animal Production and Health received an in-depth look at the U.S.
beef industry as part of the USDA Foreign Agriculture Service’s Cochran
Fellowship Program. Tiskumarage Aruni Tiskumara, DVM, who headed the delegation,
expressed confidence in the safety and quality of U.S. beef.

“We had good exposure to all of the systems the U.S. beef industry has
adopted,” she explains. “We are quite satisfied with the processing regulations
and with the biosecurity aspects as well.”

Tiskumara noted that the “negligible risk” designation for BSE from the
World Organization for Animal Health, which the U.S. received in May 2013, was a
key factor in Sri Lanka’s decision to reopen the market.

“Until the U.S. achieved negligible risk status, we were not interested in
U.S. beef,” she said. “But now that you have the negligible risk designation,
technically there is no reason why we should not import beef from the
U.S.A.”

Sri Lanka is also open to U.S. beef from cattle of all ages. Last year, Sri
Lanka imported 74 mt of beef – mostly from Australia – valued at about
$400,000.

Markets that remain closed to U.S. beef due to BSE include China,
Australia, Argentina, Brazil, Uruguay, Morocco, Israel and South Africa. Saudi
Arabia reopened to U.S. beef in 2004, but suspended imports after a BSE case was
detected in California in April 2012.

Joe Schuele is communications director for the U.S. Meat Export Federation.

Variant Creutzfeldt–Jakob disease (vCJD) has been recognized to date only
in individuals homozygous for methionine at PRNP codon 129. Here we show that
transgenic mice expressing human PrP methionine 129, inoculated with either
bovine spongiform encephalopathy (BSE) or variant CJD prions, may develop the
neuropathological and molecular phenotype of vCJD, consistent with these
diseases being caused by the same prion strain. Surprisingly, however, BSE
transmission to these transgenic mice, in addition to producing a vCJD-like
phenotype, can also result in a distinct molecular phenotype that is
indistinguishable from that of sporadic CJD with PrPSc type 2. These data
suggest that more than one BSE-derived prion strain might infect humans; it is
therefore possible that some patients with a phenotype consistent with sporadic
CJD may have a disease arising from BSE exposure.

I have been asked by Professor Collinge to respond to your request. I am a
Senior Scientist in the MRC Prion Unit and the lead author on the paper. I have
attached a pdf copy of the paper for your attention.

Thank you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you will
find in the paper, we have managed to associate the alternate phenotype to type
2 PrPSc, the commonest sporadic CJD. It is too early to be able to claim any
further sub-classification in respect of Heidenhain variant CJD or Vicky
Rimmer's version. It will take further studies, which are on-going, to establish
if there are sub-types to our initial finding which we are now reporting. The
main point of the paper is that, as well as leading to the expected new variant
CJD phenotype, BSE transmission to the 129-methionine genotype can lead to an
alternate phenotype which is indistinguishable from type 2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I can
be of any further assistance please to not hesitate to ask. Best wishes.

The TSE road map defining the evolution of European policy for protection
against prion diseases is based on a certain numbers of hypotheses some of which
may turn out to be erroneous. In particular, a form of BSE (called atypical
Bovine Spongiform Encephalopathy), recently identified by systematic testing in
aged cattle without clinical signs, may be the origin of classical BSE and thus
potentially constitute a reservoir, which may be impossible to eradicate if a
sporadic origin is confirmed.

***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases
constitute an unforeseen first threat that could sharply modify the European
approach to prion diseases.

EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far

*** but the possibility that a small proportion of human cases so far
classified as "sporadic" CJD are of zoonotic origin could not be excluded.
Moreover, transmission experiments to non-human primates suggest that some TSE
agents in addition to Classical BSE prions in cattle (namely L-type Atypical
BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic
wasting disease (CWD) agents) might have zoonotic potential.

To date the OIE/WAHO assumes that the human and animal health standards set
out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE
which include the H-type and L-type atypical forms. This assumption is
scientifically not completely justified and accumulating evidence suggests that
this may in fact not be the case. Molecular characterization and the spatial
distribution pattern of histopathologic lesions and immunohistochemistry (IHC)
signals are used to identify and characterize atypical BSE. Both the L-type and
H-type atypical cases display significant differences in the conformation and
spatial accumulation of the disease associated prion protein (PrPSc) in brains
of afflicted cattle. Transmission studies in bovine transgenic and wild type
mouse models support that the atypical BSE types might be unique strains because
they have different incubation times and lesion profiles when compared to C-type
BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian
hamster the resulting molecular fingerprint had changed, either in the first or
a subsequent passage, from L-type into C-type BSE.

***In addition, non-human primates are specifically susceptible for
atypical BSE as demonstrated by an approximately 50% shortened incubation time
for L-type BSE as compared to C-type. Considering the current scientific
information available, it cannot be assumed that these different BSE types pose
the same human health risks as C-type BSE or that these risks are mitigated by
the same protective measures.

*** Because typical clinical signs of BSE cannot always be observed in
nonambulatory disabled cattle, and because evidence has indicated these cattle
are more likely to have BSE than apparently healthy cattle, FDA is designating
material from nonambulatory disabled cattle as prohibited cattle materials.

“As of March 20, 2014, FSIS has completed all checks (effectiveness checks
and disposition verification checks) for recalls 002-2014 and 013-2014 regarding
Rancho Feeding Corporation. FSIS has determined that based on the number of
successful checks (see Directive 8080.1, Attachment 1, Table 3) where businesses
were notified of the recall and removed affected products from commerce that the
recall activities were effective.”

*** And Terry, I promised the editor you would respond so thanks for
backing my prediction up. I have read your tripe before so did not reread the
whole thing. but your point about the age of the cattle takes on the scientific
regulatory bodies of every country but one that exports US beef. They all, but
one, agree that meat from cattle under 30 months of age carries zero risk of BSE
prions. 1 △ ▽ • Reply • Share ›

Terry S. Singeltary Sr. > doc raymond • a month ago

Dr. Richard Raymond Sir, I only reply when you are scientifically wrong. I
commented today, because again, you were scientifically wrong, and I proved it
again, with scientific facts to back it up. sorry if that upsets you. you can
fool some of the folks some of the time, but not all of us all the time. you
either blatantly lied in your editorial, or you are grossly uninformed, time and
time again. I think the public can take their pick on that, and in both cases,
and they would be correct in both cases, in my opinion. you have a nice day sir.
...kind regards, terry

let's take a closer look at this new prionpathy or prionopathy, and then
let's look at the g-h-BSEalabama mad cow.

This new prionopathy in humans? the genetic makeup is IDENTICAL to the
g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like
this, ......wait, it get's better. this new prionpathy is killing young and old
humans, with LONG DURATION from onset of symptoms to death, and the symptoms are
very similar to nvCJD victims, OH, and the plaques are very similar in some
cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets
even better, the new human prionpathy that they claim is a genetic TSE, has no
relation to any gene mutation in that family. daaa, ya think it could be related
to that mad cow with the same genetic make-up ??? there were literally tons and
tons of banned mad cow protein in Alabama in commerce, and none of it
transmitted to cows, and the cows to humans there from ??? r i g h t $$$

ALABAMA MAD COW g-h-BSEalabama

In this study, we identified a novel mutation in the bovine prion protein
gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United
States of America. This mutation is identical to the E200K pathogenic mutation
found in humans with a genetic form of CJD. This finding represents the first
report of a confirmed case of BSE with a potential pathogenic mutation within
the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most
likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K
mutation.

I have been most interested to see IF the h-BSE (h-BSE or
g-h-BSEalabama???), but i have been most interested to see if in fact this
atypical h-BSE is more virulent than c-BSE, as is the L-BSE (Italian strain) has
been documented to be. We know from the studies of Kong et al that h-BSE will
transmit to TG human mice;

BSE-H is also transmissible in our humanized Tg mice.

The possibility of more than two atypical BSE strains will be
discussed.

HOWEVER, as to the virulance of it one way or the other compared to c-BSE
and or L-BSE, i don't think no one has said yet or not? interesting this debate
of the h-BSE TEXAS (2nd mad cow finally confirmed 7 months after the fact, and
an act of Congress), compared to the g-h-BSEalabama strain documented in
Alabama, that is identicle to the new human CJD in the USA that is killing the
young and old, with clinical long duration, and different symptoms in some cases
too, but not related to this ??? ALSO, this IBNC BSE, might this be the
g-h-BSEalabama strain?

THE last two mad cows documented in the USA were in Alabama and Texas, both
of which were atypical h-BSE.

SINGE then, the surveillance for TSE in cattle in the USA has been reduced
to a number of which detecting any TSE would almost impossible.

Brown, who is preparing a scientific paper based on the latest two mad cow
cases to estimate the maximum number of infected cows that occurred in the
United States, said he has "absolutely no confidence in USDA tests before one
year ago" because of the agency's reluctance to retest the Texas cow that
initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven
months later, but only at the insistence of the agency's inspector
general.

"Everything they did on the Texas cow makes everything USDA did before 2005
suspect," Brown said. ...snip...end

On June 24, 2005, the USDA announced receipt of final results from The
Veterinary Laboratories Agency in Weybridge, England, confirming BSE in a cow
that had conflicting test results in 2004. This cow was from Texas, died at
approximately 12 years of age, and represented the first endemic case of BSE in
the United States. (see Texas BSE Investigation, Final Epidemiology Report,
August 2005 External Web Site Policy PDF Document Icon (PDF – 83 KB))

ALABAMA ATYPICAL H-TYPE GENETIC BSE

On March 15, 2006, the USDA announced the confirmation of BSE in a cow in
Alabama. The case was identified in a non-ambulatory (downer) cow on a farm in
Alabama. The animal was euthanized by a local veterinarian and buried on the
farm. The age of the cow was estimated by examination of the dentition as
10-years-old. It had no ear tags or distinctive marks; the herd of origin could
not be identified despite an intense investigation (see second featured item
above and Alabama BSE Investigation, Final Epidemiology Report, May 2006
External Web Site PolicyPDF Document Icon (PDF – 104 KB)).

In August 2008, several ARS investigators reported that a rare, genetic
abnormality that may persist within the cattle population "is considered to have
caused" BSE in this atypical (H-type) BSE animal from Alabama. (See
Identification of a Heritable Polymorphism in Bovine PRNP Associated with
Genetic Transmissible Spongiform Encephalopathy: Evidence of Heritable BSE
External Web Site Policy. Also see BSE Case Associated with Prion Protein Gene
Mutation External Web Site Policy.)

On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a
presumptive diagnosis of the first known case of BSE in the United States. It
was in an adult Holstein cow from Washington State. This diagnosis was confirmed
by an international reference laboratory in Weybridge, England, on December 25.
Trace-back based on an ear-tag identification number and subsequent genetic
testing confirmed that the BSE-infected cow was imported into the United States
from Canada in August 2001. Because the animal was non-ambulatory (a "downer
cow") at slaughter, brain tissue samples were taken by USDA's Animal and Plant
Health Inspection Service as part of its targeted surveillance for BSE. However
the animal's condition was attributed to complications from calving. After the
animal was examined by a USDA Food Safety and Inspection Service (FSIS)
veterinary medical officer both before and after slaughter, the carcass was
released for use as food for human consumption. During slaughter, the tissues
considered to be at high risk for the transmission of the BSE agent were
removed. On December 24, 2003, FSIS recalled beef from cattle slaughtered in the
same plant on the same day as the BSE positive cow. (see Bovine Spongiform
Encephalopathy in a Dairy Cow - Washington State, 2003.)

OAI (Official Action Indicated) when inspectors find significant
objectionable conditions or practices and believe that regulatory sanctions are
warranted to address the establishment’s lack of compliance with the regulation.

*** An example of an OAI classification would be findings of manufacturing
procedures insufficient to ensure that ruminant feed is not co...ntaminated with
prohibited material.

Inspectors will promptly re-inspect facilities classified OAI after
regulatory sanctions have been applied to determine whether the corrective
actions are adequate to address the objectionable conditions.

Blood meal used to make cattle feed was recalled because it was cross-
contaminated with prohibited bovine meat and bone meal that had been
manufactured on common equipment and labeling did not bear cautionary BSE
statement.

The accumulation of PrPSc in the hamster tongue following i.c. inoculation
of two hamster-adapted TME strains and four hamster-adapted scrapie strains
indicates that the spread of prions to the tongue may be a common event in prion
diseases. The detection of PrPSc in axons of the tongue after i.c. inoculation
suggests that one possible route involved in the establishment of tongue
infection is axonal transport of HY TME from the brain to the tongue. In this
case, TME transport may be via the motor efferent or sensory afferent pathways
of the tongue. Prion infection of the XII nucleus, the spinal trigeminal
nucleus, or the nucleus of the solitary tract would be necessary for prions to
have access to and be transported within the tongue-associated cranial nerves.
In cases of both natural and experimental oral infection of ruminants with
scrapie and BSE, as well as in infection of deer with the CWD agent, there is
evidence for the infection of the tongue-associated brain stem nuclei (27, 48,
56, 57).

snip....

The findings of the present study, and the ability of BSE to target brain
stem regions that are synaptically connected to the tongue, indicate that the
Specified Risk Material Regulations (15), which do not completely exclude tongue
from human consumption, need to be reevaluated in order to minimize human
exposure to BSE and other prion diseases through ingestion of food products
containing tongue.

Exposure of the tongue to the prion agent during oral ingestion makes it a
potential site of agent entry and neuroinvasion, especially if lesions have
disrupted the mucosal epithelium (4). Although there are no epidemiological data
that indicate this is a common route of prion agent entry, the tongue is a
highly innervated peripheral tissue that may be a relevant site of neuroinvasion
for a subset of prion diseases of livestock in which evidence for LRS infection
is lacking.

Therefore, saliva may be one source of prions that is shed from a host and
can infect a naive host through direct or indirect contact. The spread of prions
into skeletal muscle cells via nerve fibers suggest that muscle tissue, and not
just nerves that transverse muscle, are a potential source of prion exposure
upon ingestion of food products containing meat.

Progress 12/01/05 to 11/30/09

To determine if the prion agent can directly infect epithelial cells at the
tongue mucosa we analyzed the keratin layer of the stratified squamous
epithelium (SSE). PrP-res was detected in both the keratin layer of the SSE and
the SSE of filiform papillae. PrP-res did not always colocalize with markers for
nerve fibers in these locations suggesting infection was present in epithelial
cells. These studies strongly suggest that epithelial cells in the oral mucosa
can support prion infection and shedding of the mucosa may be a source of prion
infection in saliva. We investigated infection of tongue and nasal turbinates in
over 80 ruminants infected with prion diseases. In prion-infected sheep, deer,
and elk greater than 85% of tongue and nasal turbinates were PrP-res positive.
Tongue and nasal turbinates from CWD or TME-infected cattle were examined from
over 25 animals, but we were unable to detect PrP-res in any of these samples.
These findings indicate that prion infection is present in mucosal tissues in
ruminant species in which there is horizontal transmission of prions, but not in
cattle in which there is not an endemic prion infection.

Impacts The implication from these findings is that the prion agent can
spread away from the brain into mucosa tissues in the head, specifically the
tongue and nasal cavity. Since these tissues have a mucosal surface, it may be
possible that the prion agent is shed from the tongue or nasal cavity. In the
tongue, the epithelium is continually undergoing terminal differentiation and
shedding, and is then sloughed into saliva. In the nasal mucosa, olfactory
neurons continually mature and turnover during adult life and prions may be shed
from this mucosa into nasal secretions. Therefore, saliva and nasal secretions
may be a potential source of prions that are shed from a host and can infect a
naive host through direct or indirect contact. Another implication of these
findings is that prion infection of ruminant muscle is a potential threat to
animal and human food safety. The head of ruminants is banned for human or
ruminant consumption with the exception of the tongue. Our findings indicate
that prions undergo centrifugal spread from the brainstem to the tongue and can
enter skeletal muscle cells via the neuromuscular junction and further replicate
in muscle cells. These studies suggest that tongue should also be included in
the specified risk materials in order to minimize exposure to tongue products
containing prion agent.

Experiments in prion-infected rodents have shown that the oral and nasal
mucosa, including the papillae in the tongue, can harbor prions and may act as
potential sources for the horizontal transmission of animal prion diseases since
these tissues may release prions during their normal turnover. 34,35 Equally,
the tongue, and the oral and nasal mucosa can also act as routes for
neuroinvasion.36-39 Although prions were reported previously in the saliva of
CWD-infected mule deer,10 the titers were not determined. A pooled and
concentrated (10- fold) saliva sample from five white-tailed deer with CWD was
reported to transmit prion disease to 8 of 9 Tg(CerPrP+/-)1536 mice in 342 ± 102
dpi.11 Based on published titration results with a pooled elk CWD inoculum in
Tg(CerPrP+/-)1536 mice,40 we estimated the equivalent titer for this pooled CWD
saliva sample to be -0.7 log ID50 U, which is similar to our value of -0.9 log
ID50 U for scrapie saliva sample 444 based on 30 μl of 10% saliva preparations
(Table 1).

snip...

Our results indicate a similar magnitude of salivary prion shedding between
sheep and deer. Although salivary prion titers are much lower in comparison to
those measured in brain, spleen and lymph nodes of affected animals, the number
of prions secreted in saliva over the incubation period may approach that found
in the brains of terminally ill animals, assuming that, similar to fecal prion
shedding, salivary prion shedding is not restricted to terminally sick animals
(Table 4). Also, possible differences between prion strains and animal species
used in titration studies may affect the estimated titers. Whether these
assumptions are accurate requires additional studies. It remains to be
determined at what quantities scrapie prions are shed in urine and feces of
scrapie-infected sheep (Table 4) as well as when and to what level this shedding
may occur in presymptomatic animals. Whether prions shed in saliva have
different strain characteristics from prions shed in feces is also unknown. In
light of the similarities in peripheral prion distribution patterns and shedding
of infectious prions by small ruminants and cervids, it will be important to
determine whether patients with variant Creutzfeldt-Jakob disease (vCJD) who
harbor prions in their lymphoreticular tissues,43,44 also shed vCJD prions in
saliva, urine or feces.

The present study was designed to assess the susceptibility of the
prototypic mouse line, Tg(CerPrP)1536+/- to bovine spongiform encephalopathy
(BSE) prions, which have the ability to overcome species barriers.
Tg(CerPrP)1536+/- mice challenged with red deer-adapted BSE resulted in a
90-100% attack rates, BSE from cattle failed to transmit, indicating agent
adaptation in the deer.

I lost my mother to the Heidenhain Variant of Creutzfeldt Jakob Disease DOD
12/14/97 ‘confirmed’, and never could except the science that the USDA et al
brought forth to date i.e. the UKBSEnvCJD only theory. I simply made a promise
to mom, NEVER FORGET, and so soon you all FORGOT $

Seems none of the officials remember, that went through the mad cow
debacle, or mad cow follies as I have termed them, and they are ongoing, for
anyone who thinks mad cow disease is gone, they are only fooling themselves, and
at the same time, making fools out of everyone that goes along with this TSE
prion mad cow junk science that the OIE has brought to every country around the
globe, and we will ALL pay the price in the years and decades to come. ...

IT is of my opinion, that the OIE and the USDA et al, are the soul reason,
and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion
diseases, including typical and atypical BSE, typical and atypical Scrapie, and
all strains of CWD, and human TSE there from, spreading around the globe.

I have lost all confidence of this organization as a regulatory authority
on animal disease, and consider it nothing more than a National Trading
Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i
said before, OIE should hang up there jock strap now, since it appears they will
buckle every time a country makes some political hay about trade protocol,
commodities and futures. IF they are not going to be science based, they should
do everyone a favor and dissolve there organization.

JUST because of low documented human body count with nvCJD and the long
incubation periods, the lack of sound science being replaced by political and
corporate science in relations with the fact that science has now linked some
sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of
CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call
for this organization to be dissolved. ...

IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate
declarations made by the official Veterinary Services of Member Countries. The
OIE is not responsible for inaccurate publication of country disease status
based on inaccurate information or changes in epidemiological status or other
significant events that were not promptly reported to the Central Bureau,

World Organization for Animal Health (OIE) has upgraded the United States'
risk classification for mad cow disease to "negligible" from "controlled", and
risk further exposing the globe to the TSE prion mad cow type disease

U.S. gets top mad-cow rating from international group and risk further
exposing the globe to the TSE prion mad cow type disease

*** When people talk about 1 per million, often they interpret that as
thinking it is incredibly rare. They think they have a 1-in-a-million chance of
developing this disease. You haven’t. You’ve got about a 1-in-30,000 chance of
developing it.

About Me

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS